Anomalous right or left coronary artery from the contralateral coronary sinus: “High-risk” abnormalities in the initial coronary artery course and heterogeneous clinical outcomes

doi:10.1016/S0002-8703(97)70184-4

American Heart Journal

Volume 133, Issue 4, April 1997, Pages 428-435

https://doi.org/10.1016/S0002-8703(97)70184-4 Get rights and content

Abstract

Coronary artery anomalies are associated with sudden cardiac death, although individual patient outcomes are highly variable. We performed blinded pathologic analysis of 30 consecutive cases of anomalous right ( n = 21) or left ( n = 9) coronary artery from the contralateral coronary sinus to determine which, if any, features might aid in risk stratification for sudden cardiac death. We found no significant differences in length of aortic intramural segment, coronary ostial size, degree of displacement of the anomalous coronary artery from the correct coronary sinus, or angle of coronary takeoff between patients with ( n = 12) and without ( n = 18) sudden cardiac death. All pathologic features showed considerable interpatient variability. Age ≥30 years was the only variable associated with a decreased incidence of sudden cardiac death. Thus no simple relation exists between variations in the initial coronary artery course and clinical outcome in these anomalies. (Am Heart J 1997;133:428-35.)

Section snippets

METHODS

We identified 30 consecutive (age ≥14 years) cases of anomalous right ( n = 21) and left ( n = 9) coronary artery arising from the contralateral coronary sinus with a course between the aorta and pulmonary trunk referred to the Armed Forces Institute of Pathology between 1980 and 1995. Only cases in which the coronary anomaly was an isolated finding and the intact heart, aortic root, and initial coronary artery course were available were included. The cases we report include 19 that have been

RESULTS

Table I displays data for all patients according to cause of death (anomaly-related sudden cardiac death vs other). No significant differences were found in pathologic variables, including transverse ostial dimension, angle takeoff of the anomalous coronary artery from the aorta, length of the aortic intramural coronary segment, and degree of displacement from the appropriate coronary sinus between groups of patients with and without anomaly-related sudden cardiac death (Fig. 1 , A-D ).

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Anatomic correlates of anomaly-related sudden cardiac death

Sudden cardiac death related to anomalous right or left coronary artery arising from the contralateral coronary sinus is believed to be the result of myocardial ischemia arising from one of several different anatomic abnormalities. Among these are abnormalities of the coronary ostium, specifically a narrowed, slitlike coronary orifice, and the initial coronary artery course, including an acute angle takeoff with an aortic intramural course. The importance of the slitlike orifice and acute angle

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As a result of increasing adoption of imaging screening, the number of adult patients with a diagnosis of anomalous aortic origin of the coronary arteries (AAOCA) has grown in recent years. Existing guidelines provide a framework for management and treatment, but patients with AAOCA present with a wide range of anomalies and symptoms that make general recommendations of limited applicability. In particular, a large spectrum of interventions can be used for treatment, and there is no consensus on the optimal approach to be used. In this paper, a multidisciplinary group of clinical and interventional cardiologists and cardiac surgeons performed a systematic review and critical evaluation of the available evidence on the interventional treatment of AAOCA in adult patients. Using a structured Delphi process, the group agreed on expert recommendations that are intended to complement existing clinical practice guidelines.
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As widely discussed in recent literature, coronary artery anomalies only occasionally lead to potentially serious myocardial ischemic events. The most important group of coronary anomalies has been called anomalous coronary artery origin from an abnormal sinus or a site in the ascending aorta (ACAOS). Only some cases of right- or left-sided intramural-course ACAOS (R-ACAOS-IM or L-ACAOS-IM) can potentially cause significant symptoms or sudden cardiac death, typically during exertion in athletes. After an ACAOS-IM case is qualitatively identified, it is necessary to establish the severity of associated stenosis (which is always present to some degree in ACAOS-IM). The 3 stages of a comprehensive diagnostic process are: 1. initial screening of high-risk populations (young elite athletes, optimally by use of magnetic resonance imaging [MRI]) to identify the prevalence of similar cases in large populations (the denominator of any risk calculation); 2. evaluating symptoms (chest pain, syncope, or sudden death) and performing stress testing; 3. in patients found to carry ACAOS-IM, evaluating the severity of coronary obstruction by intravascular ultrasonography, which is an objective, definitive, and quantifying imaging modality for this condition, essential in selected carriers of such anomalies. The possible treatment alternatives are discussed and updated.

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^☆: From ^aCardiology Service, Department of Medicine, Walter Reed Army Medical Center, and the ^cCardiovascular Division, Armed Forces Institute of Pathology, Washington, D.C. and the ^bDivision of Cardiology, San Francisco General Hospital.

^☆☆: Reprint requests: Renu Virmani, MD, Cardiovascular Division, Armed Forces Institute of Pathology, 14th St. and Alaska Ave. NW, Washington, DC 20307.

^★: 4/1/79277

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Anomalous right or left coronary artery from the contralateral coronary sinus: “High-risk” abnormalities in the initial coronary artery course and heterogeneous clinical outcomes☆,☆☆,★

Abstract

Section snippets

METHODS

RESULTS

Anatomic correlates of anomaly-related sudden cardiac death

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